Jing Chaoyang, Wang Zhuo, Chu Chaojia, Dong Ming, Lin Weihong
Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China.
Medicine (Baltimore). 2018 Mar;97(9):e9824. doi: 10.1097/MD.0000000000009824.
Bickerstaff brainstem encephalitis (BBE) and Miller-Fisher syndrome (MFS) might be a pedigree disease. Herein, we report a rare case that fits the diagnoses of both MFS and BBE.
A 48-year-old woman was hospitalized due to blurred vision and unsteady gait lasting for 9 days, and numbness of the limbs lasting for 6 days. Physical examination showed restricted eye movement without nystagmus, bilateral flattening of forehead and nasolabial folds, and positive eyelash sign. Her tongue deviated to the left when protruded. She had negative tendon reflex, bilateral Babinski signs, hypalgesia, and numbness in all limbs. She had positive Romberg sign and failed the right heel-knee-tibia tests. Her brain diffusion-weighted magnetic resonance imaging (DWI) showed an abnormally high circular signal in the brainstem surrounding the fourth ventricle. She also had cerebral spinal fluid (CSF) albuminocytological dissociation and GQ1b-IgG antibodies in both CSF and serum.
The case fits the diagnoses of both MFS and BBE.
The patient was treated with dexamethasone.
The condition of the patient significantly improved after the administration of dexamethasone. Her symptoms had continued to improve by the 6-week and 2-month follow-ups.
These results suggest that BBE and MFS might be a pedigree disease and timely hormone therapy is expected to improve patients'outcomes.
